Multimodality Management of Vein of Galen Malformations-An Institutional Experience

The vein of Galen aneurysmal malformation (VGM) is a rare intracranial arteriovenous fistula with a dramatic manifestation during infancy and 100% mortality without treatment. Therapeutic strategies for VGMs have changed over time as a result of advances in endovascular techniques. We present our experience and multimodality approach within the last 4 decades.

A retrospective analysis and angiographic review were performed of patients with VGM between 1975 and 2016 at our institution.

Eighteen consecutive patients were identified, including 10 with choroidal and 8 with mural VGMs. In 37 endovascular interventions, a transarterial approach was mostly performed (82.8%). One patient was initially treated surgically and received 2 Gamma Knife treatments after multiple embolizations. The preferred embolization agent was histoacryl for choroidal VGMs and a combination of coils and histoacryl for mural VGMs. Total occlusion was achieved in 87.5% of mural VGMs and 11.1% of choroidal VGMs. Cerebral hemorrhages and infarction occurred only in patients younger than 1 month. At a median follow-up interval of 4.6 years, good outcome was achieved in 53.8% and poor outcome in 46.2%, with an overall mortality of 16.7%. Poor outcome was significantly associated with choroidal-type VGMs, age <1 month at first embolization, and incomplete occlusion.

Endovascular embolization using a transarterial approach is the therapy of choice. Gamma Knife radiosurgery can be considered as second-line therapy in a multimodal approach. Whereas treatment within the first month of life is associated with the highest mortality and complications, we did not detect any severe adverse events thereafter.